Within mitochondria, certain oncoproteins or tumor suppressors such as Iso-citrate dehydrogenase NADP+1 (IDH1), Succinate dehydrogenase (SDH) & Fumarate hydratase (FHs), produce oncogenic metabolites, i.e., succinate and fumarate and 2-hydroxyglutarate, that are necessary for tumor initiation and several others affect the mitochondrial metabolic machinery directly or indirectly [43]. The gene discussed is IDH1; the disease is neoplasm.